The FUS-DDIT3 Interactome in Myxoid Liposarcoma

Yu, Jamie S.E.; Colborne, Shane; Hughes, Christopher S.; Morin, Gregg B.; Nielsen, Torsten O.

doi:10.1016/j.neo.2019.05.004

Cited by 31 publications

(39 citation statements)

References 48 publications

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“…reciprocal translocation results in MLPS, [8,11] gene ampli cation in 12q12-21 and 10p11-14 explains for WDLPS and DLPS, and an additional ampli cation in 6q23 and 1p32 is also necessary in DLPS. [8,10] Additionally, no former researches disclosed relationship between occurrence of LPS and exterior factors such as trauma or usage of drugs.…”

Section: Discussionmentioning

confidence: 99%

“…[8] Approximately 95% of MLPS patients have a t(12;16)(q13;p11) reciprocal chromosomal translocation resulting in an in-frame fusion of fused in sarcoma (FUS) to DNA damage inducible transcript 3 (DDIT3), and the remaining 5% patients exhibit a t(12;22)(q13;q12) translocation. [8,11] For PLPS, the rarest case among all subtypes, there is so far no constant chromosomal aberration or molecular alternation reported. [8] Diagnosis of LPS is currently highly dependent on pathological ndings, with CT or magnetic resonance imaging (MRI) responsible for the majority of pre-surgical diagnoses.…”

Section: Introductionmentioning

confidence: 99%

“…[8] Diagnosis of LPS is currently highly dependent on pathological ndings, with CT or magnetic resonance imaging (MRI) responsible for the majority of pre-surgical diagnoses. [11,12] Since many liposarcomas can be asymptotic before diagnosis, and symptoms, if there are any, are mainly nonspeci c such as abdominal pain or distention, pre-operative diagnosis for LPS is di cult. [2,13] Although golden standard for diagnosis remains to be biopsy, imaging is currently widely accepted means for diagnosis.…”

Section: Introductionmentioning

confidence: 99%

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Diagnosis and Prognosis of Retroperitoneum Liposarcoma: a single Asian center cohort of 57 cases

Liu

Xiao

2020

Preprint

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Background Liposarcoma is a form of malignancy found in soft tissues, mostly observed in the extremities. However, retroperitoneum liposarcoma has been seldom reported, and such diagnosis is also neglected. This study aims to present the clinical characteristics, diagnosis and prognosis of 5 different subtypes of liposarcoma and report our experience of treatment of the patients. Methods We conducted a single-center non-intervention retrospective study of 57 retroperitoneal liposarcoma patients who were admitted to Peking Union Medical College Hospital (PUMCH, Beijing, China) from December 2007 to May 2018. We collected and analyzed the demographic, clinical, imaging, histologic, therapeutic and prognostic data for these patients over a mean 4.5-year follow up period. Results Twenty-five (44%) patients were asymptomatic prior to diagnosis, with abdominal distension as the chief complaint for 18 (32%) patients and abdominal pain observed in 16 (28%) patients. Masses were evaluated by CT (n=48, 84%) or ultrasound (n=25, 44%). Laparotomy (n=52, 91%) was the preferred dominant therapeutic modality rather than laparoscopy (n=5, 9%). All patients were treated with R0 resection except 2 patients who underwent R2 resection. We conducted regular follow-up every 6 months after surgery for a mean duration of 4.5 years. Recurrence was experienced by 15 (26%) patients and a further 11 (19%) died during follow up. Conclusions Abdominal distension and pain are main chief complaints of liposarcoma. As extremity is the main location for liposarcoma occurrence, diagnosis of retroperitoneum liposarcoma is usually neglected. Since half of the patients are asymptomatic, timely diagnosis and treatment are highly dependent on regular ultrasound and CT imaging. R0 resection is the key to retroperitoneal liposarcoma treatment. Comparingly, patients underwent R2 resection, which is considered as a palliative treatment, have bad prognosis. Dedifferentiated liposarcoma, especially those with organ invasion, tend to have bad prognosis, and prognosis for well-differentiated or myxoid liposarcoma is relatively good.

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Section: Discussionmentioning

confidence: 99%

Section: Introductionmentioning

confidence: 99%

Section: Introductionmentioning

confidence: 99%

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Diagnosis and Prognosis of Retroperitoneum Liposarcoma: a single Asian center cohort of 57 cases

Liu

Xiao

2020

Preprint

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“…The FUS-DDIT3 fusion oncogene leads to the upregulation of several oncogenic pathways that have directed the investigation of several targeted therapies 66 .…”

Section: Myxoid Liposarcomamentioning

confidence: 99%

Recent advances in the understanding and management of liposarcoma

Haddox¹,

Riedel²

2021

Fac Rev

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Liposarcomas are a common subfamily of soft tissue sarcoma with several subtypes recognized by the World Health Organization: atypical lipomatous tumors (ALT)/well-differentiated liposarcoma (WDLPS), dedifferentiated liposarcoma (DDLPS), myxoid liposarcoma (MLPS), pleomorphic liposarcoma (PLPS), and myxoid pleomorphic liposarcoma (MPLPS). Despite shared adipocytic features among liposarcomas, the clinical approach to each subtype differs based on histology, location, clinical behavior, and specific oncogenic drivers. In this review, we highlight subtype-specific molecular features with the potential to generate novel therapies. We discuss recent clinical trials investigating the use of preoperative radiation therapy for retroperitoneal liposarcoma, chemotherapy, small molecule inhibitors, and innovative immunotherapy approaches and describe how we incorporate these advancements into the management of liposarcoma.

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“…The translocation that occurs in M/RCLPS produces a fusion protein containing the amino terminus of translocated in liposarcoma (TLS) to a full-length transcription factor, C/EBP homologous protein (CHOP). This translocation is also commonly referred to as FUS-DDIT3, where FUS (fused in sarcoma) corresponds to TLS and DDIT3 (DNA damage inducible transcript 3) represents CHOP [67]. Normally, CHOP is induced during stress conditions and causes growth arrest; however, the fusion of TLS prevents its normal function [68].…”

Section: Liposarcoma (Lps)mentioning

confidence: 99%

CDKs in Sarcoma: Mediators of Disease and Emerging Therapeutic Targets

Kohlmeyer

Gordon

Tanas

et al. 2020

IJMS

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Sarcomas represent one of the most challenging tumor types to treat due to their diverse nature and our incomplete understanding of their underlying biology. Recent work suggests cyclin-dependent kinase (CDK) pathway activation is a powerful driver of sarcomagenesis. CDK proteins participate in numerous cellular processes required for normal cell function, but their dysregulation is a hallmark of many pathologies including cancer. The contributions and significance of aberrant CDK activity to sarcoma development, however, is only partly understood. Here, we describe what is known about CDK-related alterations in the most common subtypes of sarcoma and highlight areas that warrant further investigation. As disruptions in CDK pathways appear in most, if not all, subtypes of sarcoma, we discuss the history and value of pharmacologically targeting CDKs to combat these tumors. The goals of this review are to (1) assess the prevalence and importance of CDK pathway alterations in sarcomas, (2) highlight the gap in knowledge for certain CDKs in these tumors, and (3) provide insight into studies focused on CDK inhibition for sarcoma treatment. Overall, growing evidence demonstrates a crucial role for activated CDKs in sarcoma development and as important targets for sarcoma therapy.

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The FUS-DDIT3 Interactome in Myxoid Liposarcoma

Cited by 31 publications

References 48 publications

Diagnosis and Prognosis of Retroperitoneum Liposarcoma: a single Asian center cohort of 57 cases

Diagnosis and Prognosis of Retroperitoneum Liposarcoma: a single Asian center cohort of 57 cases

Recent advances in the understanding and management of liposarcoma

CDKs in Sarcoma: Mediators of Disease and Emerging Therapeutic Targets

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